Vasculitis is a heterogeneous disease characterized by inflammation and a modification of the vessel wall affecting all tunics. The classification criteria, not to be confused with diagnostic criteria, are essential for understanding this family of diseases.

Determine the epidemiological, clinical, paraclinical and therapeutic profile of systemic vasculitis.

This is a retrospective study with descriptive purpose that involves 42 patients with systemic vasculitis.

The mean age of our patients was 44.64 ± 15.9 years, with extremes ranging from 18 to 87 years. The sex ratio men/women was 1.2 (23 men/19 women). The personal history was dominated by diabetes, HTA and atopy. The types of systemic vasculitis were as follows: 22 cases of Behçet disease, 7 cases of Takayasu disease, 6 cases of gigantocellular arteritis, 5 cases of vasculitis of small vessels, one case of lupus vasculitis and one case of leucocytoclasic cutaneous vasculitis.

The most common clinical manifestations were vascular (45.2%), mucocutaneous and articular (40.5%). An inflammatory syndrome was present in 33.3% of our patients. Immunological tests were performed in 25 patients, and were positive in 32% of the cases. Oral corticosteroid was administered in 36 patients (85.7%), immunosuppressant in 21 patients (50%) and biotherapy in 4 patients (9.5%).

Few studies describing the epidemiology of systemic vasculitis have been published, particularly regarding their incidence and prevalence. Most available data come from reference centers or very small regions, which may introduce selection bias. That said, an increase in incidence has been observed for most types of systemic vasculitis. Their clinical presentation is highly variable and polymorphic, with a wide range of manifestations.

Complementary investigations are performed to support the diagnosis of vasculitis, assess target organ involvement, identify secondary forms, and guide invasive tests and biopsies the latter being the only definitive diagnostic confirmation.

A systemic vasculitis continue to cause significant morbidity and mortality, despite the use of sometimes intensive treatments. Corticosteroid therapy, immunosuppressive agents, and biologic therapies remain the mainstays of treatment.

The clinical polymorphism of vasculitis depends on the size and distribution of the affected vessels, with varying prognostic and therapeutic implications.